TY - JOUR
T1 - Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis
AU - White, Barbara
AU - Moore, Wendy C.
AU - Wigley, Fredrick M.
AU - Xiao, Hui Qing
AU - Wise, Robert A.
PY - 2000/6/20
Y1 - 2000/6/20
N2 - Background: Lung inflammation (alveolitis) may cause lung fibrosis in scleroderma. Objective: To determine whether cyclophosphamide treatment is associated with retention of lung function and improved survival in scleroderma patients with alveolitis. Design: Retrospective cohort study. Setting: Johns Hopkins and University of Maryland Scleroderma Center. Patients: 103 patients with scleroderma who had bronchoalveolar lavage or lung biopsy. Intervention: Cyclophosphamide therapy. Measurements: 1) Serial measurement of forced vital capacity (FVC) and carbon monoxide diffusing capacity and 2) survival. Results: During a median follow-up of 13 months after bronchoalveolar lavage or biopsy, patients with alveolitis who did not receive cyclophosphamide therapy experienced a decrease in FVC (mean difference, -0.28 L [95% CI, -0.41 to -0.16 L] and -7.1% of the predicted value [CI, -10.9% to -4.0%]). Carbon monoxide diffusing capacity also decreased in these patients (mean difference, -3.3 mmol · min-1 · kPa-1 [CI, -4.6 to -2.1 mmol · min-1 · kPa-1] and -9.6% of the predicted value [CI, -16.7% to -2.4%]). During a median follow-up of 16 months, patients with alveolitis who received cyclophosphamide were more likely to have a good outcome (stabilization or improvement) in FVC (relative risk, 2.5 [CI, 1.5 to 4.1]) and diffusing capacity (relative risk, 1.5 [CI, 1.0 to 2.2]). These patients also had improved survival; the median survival rate was 89% (25th, 75th percentiles, 84%, 94%) compared with 71% (25th, 75th percentiles, 55%, 86%) in untreated patients (P = 0.01, log-rank test). Conclusions: The presence of lung inflammation identifies patients with scleroderma who are more likely to have worsening lung function. Lung function outcomes and survival are improved in patients with alveolitis who receive cyclophosphamide.
AB - Background: Lung inflammation (alveolitis) may cause lung fibrosis in scleroderma. Objective: To determine whether cyclophosphamide treatment is associated with retention of lung function and improved survival in scleroderma patients with alveolitis. Design: Retrospective cohort study. Setting: Johns Hopkins and University of Maryland Scleroderma Center. Patients: 103 patients with scleroderma who had bronchoalveolar lavage or lung biopsy. Intervention: Cyclophosphamide therapy. Measurements: 1) Serial measurement of forced vital capacity (FVC) and carbon monoxide diffusing capacity and 2) survival. Results: During a median follow-up of 13 months after bronchoalveolar lavage or biopsy, patients with alveolitis who did not receive cyclophosphamide therapy experienced a decrease in FVC (mean difference, -0.28 L [95% CI, -0.41 to -0.16 L] and -7.1% of the predicted value [CI, -10.9% to -4.0%]). Carbon monoxide diffusing capacity also decreased in these patients (mean difference, -3.3 mmol · min-1 · kPa-1 [CI, -4.6 to -2.1 mmol · min-1 · kPa-1] and -9.6% of the predicted value [CI, -16.7% to -2.4%]). During a median follow-up of 16 months, patients with alveolitis who received cyclophosphamide were more likely to have a good outcome (stabilization or improvement) in FVC (relative risk, 2.5 [CI, 1.5 to 4.1]) and diffusing capacity (relative risk, 1.5 [CI, 1.0 to 2.2]). These patients also had improved survival; the median survival rate was 89% (25th, 75th percentiles, 84%, 94%) compared with 71% (25th, 75th percentiles, 55%, 86%) in untreated patients (P = 0.01, log-rank test). Conclusions: The presence of lung inflammation identifies patients with scleroderma who are more likely to have worsening lung function. Lung function outcomes and survival are improved in patients with alveolitis who receive cyclophosphamide.
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U2 - 10.7326/0003-4819-132-12-200006200-00004
DO - 10.7326/0003-4819-132-12-200006200-00004
M3 - Article
C2 - 10858177
AN - SCOPUS:0034691244
SN - 0003-4819
VL - 132
SP - 947
EP - 954
JO - Annals of internal medicine
JF - Annals of internal medicine
IS - 12
ER -